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Table 1 Mouse models for laminin α2 chain deficiency.

From: Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies

Mouse model Mutation/protein product Phenotype Ref
dy/dy Unknown spontaneous mutation/reduced expression of seemingly normal α2 chain Lethal within 6 months of age. Moderate muscular dystrophy; peripheral neuropathy; defective central nervous system myelination; hearing loss; aberrant thymocyte development [11, 12, 84, 121, 122]
dy2J/dy2J Spontaneous mutation in LN domaina/slightly reduced expression of truncated α2 chain devoid of LN domain Normal lifespan. Mild muscular dystrophy; peripheral neuropathy [85, 86]
dyW/dyW Knock- out/severely reduced expression of truncated α2 chain devoid of LN domain Lethal at 10 to 15 weeks of age. Severe muscular dystrophy; peripheral neuropathy [43, 83]
dy3K/dy3K Knock-out/complete deficiency Lethal at 4 weeks of age. Severe muscular dystrophy; peripheral neuropathy; impaired spermatogenesis; defective odontoblast differentiation [42, 123125]
dynmj417/dynmf417 N-ethyl-N-nitrosourea-induced point mutation in LN domain/normal levels Normal lifespan. Mild muscular dystrophy; peripheral neuropathy [87]
dyPas/dyPas(now extinct) Spontaneous retrotransposal insertion/severe deficiency Died at 13 weeks of age. Severe muscular dystrophy; peripheral neuropathy [126]
  1. aLN = laminin N-terminal domain.